Endgames Case Report

A woman with periodic chest pain

BMJ 2012; 345 doi: http://dx.doi.org/10.1136/bmj.e6869 (Published 29 October 2012) Cite this as: BMJ 2012;345:e6869
  1. A J Patel, foundation year 1 doctor1,
  2. R Som, general surgery specialist trainee year 32,
  3. G K Soppa, academic clinical lecturer, cardiothoracic surgery3,
  4. E E J Smith, consultant cardiothoracic surgeon3
  1. 1Maidstone Hospital, Maidstone, UK
  2. 2John Radcliffe Hospital, Oxford, UK
  3. 3Department of Cardiothoracic Surgery, St George’s Hospital, London, UK
  1. Correspondence to: R Som, 46 Whitehouse Way, London N14 7LT, UK rsom{at}doctors.org.uk

A 42 year old woman presented to the emergency department with a four hour history of central chest pain and breathlessness on exertion. She had been having similar chest pains on a periodic basis over the past year. Eight months before presentation, she had had a pneumothorax, which had not responded to chest aspiration or chest drain insertion, and had therefore undergone surgical pleurodesis.

On examination her breath sounds at the right base were reduced, with a hyper-resonant percussion note. She appeared comfortable at rest, with a respiratory rate of 16 breaths/min. Her blood pressure was 110/50 mm Hg and her heart rate was 60 beats/min, in sinus rhythm. Chest radiography was performed.

Questions

  • 1 If this were the patient’s first pneumothorax, how would you manage it acutely?

  • 2 What surgical procedure is used to treat recurrent pneumothorax?

  • 3 What underlying conditions might have caused recurrent pneumothorax in this patient?

Answers

1 If this were the patient’s first pneumothorax, how would you manage it acutely?

Short answer

For a first presentation of pneumothorax, if no underlying lung disease is evident clinically or radiologically, management depends on the size of the pneumothorax on chest radiography—if 2 cm or greater, needle aspiration should be attempted. If less than 2 cm, patients may be discharged with advice to seek urgent medical help if symptoms worsen. Patients should be followed up in two to four weeks in an outpatient setting.

Long answer

British Thoracic Society guidelines for spontaneous pneumothorax stipulate that primary spontaneous pneumothorax needs to be distinguished from secondary spontaneous pneumothorax to guide appropriate management.1 Secondary spontaneous pneumothorax occurs in the presence of underlying lung pathology. Active management is needed for all patients with pneumothorax who have severe breathlessness and for those with bilateral and tension pneumothoraxes.1 The guidelines also state that a pneumothorax of 2 cm will occupy 49% of the hemithorax and that such a pneumothorax therefore requires intervention.1 Our patient’s vital signs and subsequent investigations are consistent with haemodynamic stability, but because the pneumothorax was 2 cm (fig 1), aspiration with a 16-18G cannula should be undertaken. Insertion of a chest drain is indicated if initial attempts at aspiration are unsuccessful. At this stage, no underlying lung disease had been identified, so this was classified as a recurrent primary spontaneous pneumothorax.

Figure1

Fig 1 Chest radiograph showing a pneumothorax of roughly 2 cm in diameter

2 What surgical procedure is used to treat recurrent pneumothorax?

Short answer

Patients should be considered for pleurodesis using video assisted thoracic surgery (VATS).

Long answer

Surgical pleurodesis—through a VATS or open approach (thoracotomy)—is superior to medical pleurodesis in terms of preventing recurrences.1 Patients should be urgently referred to the thoracic surgeons for VATS if a pneumothorax does not resolve within three to five days.1 Surgical pleurodesis has two aims—to resect visible bullae or repair porosities on the visceral pleura, and to form a symphysis between the opposing pleural surfaces to prevent further recurrence. This can be achieved by pleural abrasion and varying degrees of pleurectomy; in reality a combination of the two methods is used.1 The minimally invasive VATS approach is popular because morbidity is lower, although recurrence is higher than with the open approach—5% and 1%, respectively.2 VATS has the added benefit of potentially diagnosing underlying conditions, either macroscopically or by enabling biopsies to be taken for histology. It can also be used for additional treatments, such as resection of underlying disease processes. For patients who are unwilling or unfit to undergo surgery, medical pleurodesis can be achieved by infiltration of sclerosing agents into the pleura through a chest drain.

3 What underlying conditions might have caused recurrent pneumothorax in this patient?

Short answer

Cancer, connective tissue disorders, and thoracic endometriosis syndrome are potential differential diagnoses in this patient. Chronic obstructive pulmonary disease, asthma, and cystic fibrosis are also important causes of secondary spontaneous pneumothorax, although they are unlikely in this patient.

Long answer

Pneumothorax can be classified as spontaneous (primary or secondary), traumatic, or iatrogenic.3

Primary spontaneous pneumothorax typically occurs in tall thin males aged 10-30 years.4 Despite the absence of underlying lung pathology, subpleural bullae are seen in 76-100% of these patients during VATS exploration.5 6 The mechanism of bullae formation may be related to smoking induced free radical release, neutrophil dysfunction, and destruction of pulmonary parenchyma as a result of protease release.7 8

Secondary spontaneous pneumothorax is associated with a greater risk of death than primary disease, given the underlying lung disease and limited cardiopulmonary reserve.3 Recurrence rates are similar in both types of disease, at 39-47%.8

Most cases of secondary spontaneous pneumothorax are caused by chronic obstructive pulmonary disease, asthma, and cystic fibrosis. The severity of chronic obstructive pulmonary disease positively correlates with an increased risk of subsequent pneumothorax.3 Given our patient’s age and history, however, none of these conditions is likely to be the underlying problem. The box lists the underlying conditions that should be considered.

Underlying conditions associated with secondary spontaneous pneumothorax

Airway disease
  • Chronic obstructive pulmonary disease

  • Asthma

  • Cystic fibrosis

Infectious lung disease
  • Pneumocystis carinii pneumonia

  • Necrotising pneumonia

  • Tuberculosis

Interstitial lung disease
  • Sarcoidosis

  • Idiopathic pulmonary fibrosis

  • Langerhans cell histiocytosis

  • Tuberous sclerosis

Connective tissue disorders
  • Rheumatoid arthritis

  • Ankylosing spondylitis

  • Polymyositis

  • Dermatomyositis

  • Scleroderma

  • Marfan’s syndrome

  • Ehlers-Danlos syndrome

Cancer
  • Sarcoma

  • Primary lung cancer

  • Metastatic lung cancer

Familial
  • Birt-Hogg-Dube syndrome

Miscellaneous
  • Thoracic endometriosis syndrome

  • Lymphangioleiomyomatosis

Connective tissue disorders (rheumatoid arthritis, scleroderma, Ehlers-Danlos syndrome) and cancer should be considered, particularly in younger patients without the risk factors mentioned above. The most common cancers are primary lung tumours or metastatic lung disease from breast tissue. A retrospective study carried out on 1199 cases of pneumothorax found that 93 of the 505 people with secondary disease had a primary or metastatic tumour.9 Diagnosis is typically based on histological findings—on skin for connective tissue disorder and lung lesions for cancer.

Infections, particularly HIV associated Pneumocystis carinii pneumonia, which has a high mortality,10 are strongly associated with secondary pneumothorax. Interstitial lung diseases such as sarcoidosis or idiopathic pulmonary fibrosis should be considered in younger patients.

Rarer associations with secondary spontaneous pneumothorax seen only in women of childbearing age are catamenial pneumothorax and lymphangioleiomyomatosis. Catamenial pneumothorax is defined as spontaneous recurrent pneumothorax that occurs within 72 hours before or after the onset of menstruation.11 It is most common in women with thoracic endometriosis syndrome.12 The reasons for this are unclear, although there are several hypotheses.11 12 The incidence of thoracic endometriosis as a cause of pneumothorax may be grossly underestimated. Lymphangioleiomyomatosis affects women of reproductive age. It is characterised by the proliferation of smooth muscle cells along lymphatic channels, and pneumothorax develops in 80% of women known to have the disease.13 Both of these conditions are amenable to hormonal therapy.

Patients with a family history of pneumothorax may have a genetic disorder, such as Birt-Hogg-Dube syndrome.14 The underlying disease is a result of germline mutations in the folliculin gene, which clinically manifest as pneumothoraxes, skin fibrofolliculomas, pulmonary cysts, or renal cancer.14

Patient outcome

In keeping with British Thoracic Society guidelines, needle aspiration was used in an attempt to reduce the pneumothorax to less than 1 cm, but neither this nor insertion of a chest drain was successful. The patient therefore underwent a second pleurodesis as an inpatient and was referred for a gynaecology opinion because both pneumothoraxes had coincided with menstruation. A diagnostic laparoscopy showed pelvic endometriosis. Computed tomography showed right sided pulmonary nodules that were in keeping with endometrial deposits (fig 2) This confirmed that thoracic endometriosis syndrome had caused catamenial pneumothoraxes. Treatment was started with leuprorelin, an agonist of gonadotrophin releasing hormone. However, she has since had another pneumothorax and is being considered for hysterectomy with bilateral salpingo-oophorectomy.

Figure2

Fig 2 Computed tomogram showing a right sided pneumothorax (arrow) with multiple ipsilateral pleural nodules on the surface of the diaphragm (red arrowheads)

Notes

Cite this as: BMJ 2012;345:e6869

Footnotes

  • Competing interests: All authors have completed the ICMJE uniform disclosure form at www.icmje.org/coi_disclosure.pdf (available on request from the corresponding author) and declare: no support from any organisation for the submitted work; no financial relationships with any organisations that might have an interest in the submitted work in the previous three years; no other relationships or activities that could appear to have influenced the submitted work.

  • Provenance and peer review: Not commissioned; externally peer reviewed.

  • Patient consent obtained.

References